Copyright © by ESPEN LLL Programme 2016

Nutritional Support

in Neurological Diseases Topic 25

Module 25.4

Nutritional Support in Chronic Neurological Diseases

Rosa Burgos

Endocrinology and Nutrition

Nutritional Support Unit

University Hospital Vall d’Hebron

Pg. Vall d’Hebron 117-127

08035, Barcelona. Spain

Learning Objectives

To know about nutritional intervention in patients suffering from multiple sclerosis or Parkinson’s disease;

To know the problems of clinical decision making in the timing/safety/efficacy of

percutaneous endoscopic gastrostomy (PEG) tube feeding in amyotrophic lateral sclerosis (ALS) patients;

To understand the clinical challenges of establishing and maintaining tube feeding in neurological patients with advanced dementia;

To discuss the indications for gastrostomy in neurological patients with advanced dementia;

To know the ethical, legal, and moral implications of nutritional support in patients with neurodegenerative disorders with progressive dementia.

Contents 1. Introduction

2. Nutritional support in chronic neurological conditions 2.1. Multiple sclerosis

2.2. Parkinson’s disease 2.3. Neuromuscular disease (including amyotrophic lateral sclerosis)

3. Enteral feeding: considerations in patients with chronic neurological disorders

4. Tube feeding / PEG placement in neurodegenerative disorders 5. Ethical considerations: tube feeding in neurodegenerative disorders with advanced

dementia 6. Summary

7. References

Key Messages Patients with chronic neurological diseases are at risk of malnutrition, with dysphagia

the most frequent concern in terms of evaluation and treatment;

Amyotrophic lateral sclerosis patients generate the most frequent dilemmas concerning timing/efficacy and security of PEG placement;

There are no robust data available to support tube feeding over oral feeding in advanced terminal dementia;

Patients with advanced dementia are not usually considered appropriate candidates for artificial feeding via PEG tubes;

Legally and ethically, specialized nutritional support should be considered to be a medical therapy;

Advanced directives help to resolve conflicts over desired treatments.

Copyright © by ESPEN LLL Programme 2016

1. Introduction Neurodegenerative diseases are a heterogeneous group of disorders that are characterized

by the progressive degeneration of the structure and function of the central nervous system (CNS) or peripheral nervous system.

The CNS acts as a regulator of nutrient intake by internal signalling mechanisms, which

become disrupted in CNS injury. Chronic, degenerative diseases of the brain can also cause significant complications affecting the nutritional status, which develop gradually as the

disease progresses. Residual neurological function often changes over time, generating different nutritional concerns depending on the stage of CNS injury (1). Dysphagia is the

most frequent concern in terms of evaluation and treatment (2). Nutritional support should be considered as a valuable adjunctive therapy in chronic

neurological patients with malnutrition or risk of malnutrition. The objectives of the nutritional support can vary widely among diseases. In multiple sclerosis or Parkinson’s

disease the objective can be to cover all the nutritional requirements of the patient. In

chronic neurological diseases with advanced dementia like Alzheimer’s disease, the objective may be comfort feeding and reinforcement of quality of life. Nevertheless, a great

concern exists about the possibility of preventing or delaying the progression of chronic neurological disease with adequate nutritional support (for more details, see the LLL

Module 20.4)

2. Nutritional Support in Chronic Neurological Conditions

2.1 Multiple Sclerosis

Multiple sclerosis (MS) is the most common degenerative auto-immune disorder of the CNS. In the acute phase of MS, T-lymphocytes attack oligodendrocytes and this leads to

inflammation and scarring of the myelin sheaths. The conduction of nerve impulses along the axon of the neurone may be affected during an acute inflammatory phase (relapse),

but tends to improve with healing during the remission phase. Over time, relapses cause

extensive damage and scarring of the myelin sheath with progressive loss of neuronal function. The cause of MS is unknown, but research suggests that genetic, immunological

and environmental factors, such as a common virus, may all be involved in a complex aetiology (3).

Symptoms of MS vary widely between individuals, depending on the site of scarring, and can affect brain, peripheral nerves and spinal cord.

There is no direct evidence that nutrition is involved either in the aetiology of MS or in the rate of disease progression. Epidemiological studies have shown that MS is more prevalent

in countries with a high intake of saturated fat, and lower in countries with high intakes of

polyunsaturated fat. However, intervention studies using modifications in fat intake as well as modulation of n-6 and n-3 fatty acids to try and reduce the rate and severity of relapse

in MS have shown conflicting results (4). Antioxidants to inhibit oxidation of essential fatty acids by free radicals in the membrane

phospholipids of the myelin sheath have also been proposed. Although there is a growing interest in dietary antioxidants and disease activity in MS, there is no scientific evidence

that any form of dietary intervention is effective in slowing the rate or severity of this disease. Although initially a favourable relationship was postulated, a meta-analysis has

been published highlighting the lack of robust evidence on the use of vitamin D and multiple

sclerosis relapses (5). Patients with MS are at risk of malnutrition as the degree of disability progresses. For more

details, consult module 25.1.

Nutrition management

Overweight and obesity can also occur initially due to reduced mobility, corticosteroids, antidepressants drugs, and unhealthy dietary habits (use of fatty foods, sugary drinks,

Copyright © by ESPEN LLL Programme 2016

etc.) In these cases, a review of dietary habits provides the opportunity to reinforce healthy eating advice (4).

With increasing disability, weight loss, malnutrition and cachexia are frequent. Although the incidence of malnutrition in MS patients has not been determined, it can adversely

affect organ function, muscle strength and immune function, thereby worsening the symptoms of MS (6). The possible causes of malnutrition are summarized in Table 1.

Dietary management of weight loss depends upon identification of the source of the

problem.

Table 1 Aetiology of weight loss and related dietary interventions in patients

with multiple sclerosis

CAUSE INTERVENTION

Reduced mobility and fatigue Difficulty with shopping and cooking

Assistance with shopping and cooking

Physical difficulty manipulating food or

fluid to mouth (tremor or postural

difficulty)

Finger foods, lightweight utensils, special

feeding utensils

Poor sight Assistance with shopping and cooking, and with eating and drinking

Quickly fatigued when eating, so small

meals and few between-meal snacks

Small frequent meals and between-meal

snacks. Fortify foods. Dietary supplements

Poor appetite Consider side-effects of drug treatment.

Small frequent meals and snacks. Fortify

foods. Dietary supplements

Reduced cognition Extra assistance

Dysphagia Modified textured diets / EN

High dependence on reduced-fat convenience meals

Liberal use of full-fat dairy products, increase snacks rich in vegetable oils

Adverse effect of drugs Evaluate if all are needed

Dysphagia is a symptom of chronic progressive MS. The reported incidence ranges between

3 and 43% depending on the method used to evaluate the swallowing difficulty (7-10). It is often accompanied by speech difficulties. Features of dysphagia include coughing and

choking during meals, frequent chest infections and weight loss. The evaluation of the ability to swallow may be clinical initially but this should be followed by video fluoroscopy

or fibreoptic endoscopic evaluation of swallowing. The type of nutritional intervention depends on the degree of difficulty, and ranges between modified consistency diets and

exclusive artificial nutrition if the risk of aspiration is very high.

If weight loss progresses despite oral intervention or if the dysphagia cannot be treated safely by dietary modifications, enteral nutrition via a PEG should be considered. Enteral

tube feeding can improve nutritional status, reduce the risk of aspiration pneumonia, reduce the risk of pressure sores, and maintain fluid balance as well as allowing some

continuing oral intake. However, before embarking on treatment, the pros and cons of artificial nutrition should be discussed fully with the patient and family.

2.2 Parkinson’s Disease

Parkinson’s disease (PD) is a chronic, progressive neurodegenerative disorder resulting from dopamine depletion in the brain. The main symptoms include tremor, muscular

rigidity, bradykinesia and postural instability. As PD progresses, a variety of other symptoms emerge, including dysphagia, dysarthria, impaired gastrointestinal motility,

fatigue, depression and cognitive impairment. Drug therapy is essential to control

symptoms and to maintain mobility in PD, and acts by replacing or mimicking dopamine in the brain.

Copyright © by ESPEN LLL Programme 2016

Patients with PD are at increased risk of malnutrition and weight loss (11), and nutritional status should be monitored routinely regularly throughout the natural history of the disease

(12, 13) (see module 25.1). Dysphagia in PD usually occurs in the advanced phases of the disease, although sometimes

it is present from the onset. Functional alterations in oropharyngeal and oesophageal motility can be present in about 60-80% of patients, but may be asymptomatic.

Impaired gastric emptying is commonly reported in patients with PD at both early and

advanced stages. It seems to be related to severe motor function impairment and motor fluctuations, typical of disease progression. It can be treated with domperidone.

Malnutrition in PD patients is probably under-reported, and can be found in about 15% in community-dwelling patients with PD, and another 24% of patients are at medium or high

risk of malnutrition. Several predictors of malnutrition have been found: older age at diagnosis, higher levodopa equivalent daily dose / body weight, greater UPDR score,

anxiety and depression, and living alone (13). Amino acids compete with levodopa for transport across the blood-brain barrier, and it was

therefore postulated that low protein diets might improve drug uptake and enhance

mobility in patients taking levodopa. However, dietary protein restriction seems to be helpful in only a small number of patients who have severe medication-induced fluctuations

in mobility, and is not recommended for continuous use. Rather than restricting protein, patients should be advised to redistribute protein throughout the day and to avoid taking

high protein foods at the same time as their levodopa. With the introduction of controlled release preparations of levodopa the proportion of patients with PD who might benefit from

protein restriction has declined. In severe cases, duodenal infusion of levodopa (duodopa) can be explored (14, 15).

In summary, in PD our primary nutrition intervention will be focused on healthy dietary

intake and minimising adverse drug-nutrient interactions to avoid weight loss and to manage malnutrition and dysphagia.

2.3 Neuromuscular Diseases (Amyotrophic Lateral Sclerosis)

Malnutrition is a common problem among patients with neuromuscular diseases, particularly in those with motor neurone disease. Amyotrophic lateral sclerosis (ALS) is a

prototype model of neurodegenerative disease in this respect, characterized by the progressive loss of motor neurones, resulting in the progressive wasting of skeletal

muscles, including the respiratory muscles. The recognition of nutrition as an independent, prognostic factor for survival and disease

complications in ALS has highlighted the importance of the nutritional management of these patients (16,17). Patients who have lost weight at diagnosis have a doubled risk of

death, and malnourished patients show a 7.7-fold increase in mortality (17).

The aetiology of malnutrition in ALS patients is multifactorial (16): - The bulbar degeneration of neurones manifests as difficulties with mastication,

impaired oral transit, prolonged meal times and dysphagia. - Anorexia is frequent and commonly attributed to psychological distress, depression and

polypharmacy. - The weakening of abdominal and pelvic muscles, limited physical activity, self

restriction of fluids and lower-fibre diets can result in constipation, which in turn can exacerbate the anorexia.

Despite the reduction in lean body mass, ALS patients have increased energy needs, due

to increased respiratory efforts, pulmonary infections and other factors yet not established (18). Among patients suffering from respiratory failure, non-invasive home ventilation is

commonly practiced in most major European centres, and this may mean that energy requirements need reassessment to avoid weight loss and net catabolism, and also because

of the effects of carbohydrate and protein intake on metabolic rate and the demands for increased gas exchange.

Malnutrition exacerbates the disease related breakdown and atrophy of skeletal and respiratory muscles, and impairs the immune system, predisposing to infection, a common

cause of death in ALS patients (17).

Copyright © by ESPEN LLL Programme 2016

The key issues for nutritional management in amyotrophic lateral sclerosis include (19,20):

- Energy supplementation - Diagnosis/treatment of dysphagia

- Timing/safety/efficacy of PEG tube placement.

Energy supplementation

Recently, NICE Guidelines on motor neurone disease have been published (21), and some recommendations are summarized about oral nutritional support in ALS.

1. At diagnosis and regularly, we need to assess the person’s weight, personal diet, nutritional and fluid intake, hydration, oral health, feeding, drinking and swallowing,

and offer support, advice and interventions as needed. To design nutritional intervention, we need to assess appetite and thirst, gastrointestinal symptoms, such

as nausea or constipation, and evaluate causes of reduced oral intake (for example, swallowing difficulties, limb weakness or the possibility of low mood or depression

causing loss of appetite.

2. Assess the person’s ability to eat and drink by taking into account:

a. the need for eating and drinking aids and altered utensils to help them take food

from the plate to their mouth.

b. the need for help with food and drink preparation.

c. advice and aids for positioning, seating and posture while eating anddrinking.

d. dealing with social situations (for example, eating out).

3. Arrange for a clinical swallowing assessment if swallowing problems are suspected.

4. Assess and manage factors that may contribute to problems with swallowing, such as:

positioning, seating, the need to modify food and drink consistency and palatability, respiratory symptoms and risk of aspiration and/or choking, and fear of choking and

psychological considerations (for example, wanting to eat and drink without assistance

in social situations).

When considering diet, although the available evidence showed no benefit of supplements in terms of survival, food supplementation may be useful for some people and should be

considered on an individual basis following a nutritional assessment. The NICE Guidelines identified that it was preferable to increase calorie intake by enriching food instead of

providing food supplements.

Diagnosis and treatment of dysphagia

Dysphagia in ALS patients is highly prevalent, and can be the initial symptom in bulbar

onset ALS. In advanced forms of the disease, the prevalence of dysphagia can be near 100%. For more details about diagnosis of dysphagia, refer to module 25.1.

Gastrostomy

Mortality in ALS patients undergoing PEG tube insertion is strongly correlated with respiratory function. Practice guidelines from the American Academy of Neurology

recommend that the procedure should be performed when a patient’s forced vital capacity is still greater than 50% of the predicted value (for more information about the placement

of PEG tube see module 25.2). Recent reports show, however, that many PEGs have been

placed in patients who have not met this criterion (22), and several studies have proved that lower vital capacity at the time of PEG insertion is not associated with poor outcome

Copyright © by ESPEN LLL Programme 2016

when non-invasive positive pressure ventilation is used or if the gastrostomy procedure is carried out radiologically. Patients may benefit from an open discussion of the benefits and

hazards of PEG early in the course of the disease and before swallowing problems have developed. Changes in nutritional status and the identification of unintentional weight loss

or dysphagia are the best guides to the timing of PEG placement so as to maximize quality of life. A Cochrane systematic review of the literature showed that there is no evidence for

efficacy of enteral feeding in ALS patients in terms of mortality, although there are possible

advantages in other respects: of nutritional status (only one prospective study) and quality of life (understudied) (20). Probably, these results are a reflection of the lack of sufficient

randomized controlled trials due in turn to ethical difficulties and problems of study design. The American Academy of Neurology, Quality Standards Subcommittee, has published an

evidence-based review with regard to drugs, nutrients and respiratory therapies for patients with ALS (22). It was concluded that PEG feeding should be considered to stabilize

weight and to prolong survival in patients with ALS (Level B). A nutrition management algorithm is proposed (Fig. 1).

Fig. 1 Nutrition management algorithm for patients with ALS (10)

The NICE Guidelines 2016 for motor neurone disease (MND) / ALS (21) recommend:

to discuss gastrostomy at an early stage, and at regular intervals as MND progresses, taking into account the person’s preferences and issues, such as ability to swallow,

weight loss, respiratory function, effort of feeding and drinking and risk of choking. The

early placement of a gastrostomy can help to ease a person’s discomfort during the progression of MND by preventing malnutrition through enteral administration of fluids,

Copyright © by ESPEN LLL Programme 2016

food and medication. This may prolong survival. Be aware that some people will not want to have a gastrostomy.

Explain the benefits of early placement of a gastrostomy, and the possible risks of a late gastrostomy (for example, low critical body mass, respiratory complications, risk

of dehydration, different methods of insertion, and a higher risk of mortality and procedural complications).

If a person is referred for a gastrostomy, it should take place without unnecessary

delay.

Pay particular attention to the nutritional and hydration needs of people with MND who

have frontotemporal dementia and who lack mental capacity. The multidisciplinary team assessment should include the support they need from carers, and their ability to

understand the risks of swallowing difficulties.

Before a decision is made on the use of gastrostomy for a person with MND who has

frontotemporal dementia, the neurologist from the multidisciplinary team should assess the following:

o The person’s ability to make decisions and to give consent.

o The severity of frontotemporal dementia and cognitive problems.

o Whether the person is likely to accept and cope with treatment.

Discuss with the person’s family members and/or carers (as appropriate; with the person’s consent if they have the ability to give it).

3. Enteral Feeding Considerations in Patients with Chronic Neurological Disorders

Several questions need to be addressed when considering the enteral nutrition of patients with chronic neurological diseases (23):

1. Patient’s level of consciousness 2. Can the patient protect the airway from episodes of gastro-oesophageal reflux?

3. What is the patient’s ability to swallow liquids, thickened liquids, and soft and solid foods?

4. Is the patient at high risk of aspirating oropharyngeal secretions?

5. Will the need for enteral access be short- or long-term? 6. What are the nutrient requirements of patients with specific conditions (such as

ALS)? 7. Is there a difference between gastric and jejunal feeding in this population?

8. What are the patient’s and family’s desires with regard to nutritional treatment?

All these questions can help us to decide the most appropriate form of nutritional support in chronic neurological patients and how to prevent the most serious complications such

as bronchial aspiration. The causes are summarised in (Fig. 2).

Copyright © by ESPEN LLL Programme 2016

Fig. 2 Mechanisms involved in risk of bronchial aspiration

in chronic neurological patients with enteral nutrition.

4. Tube Feeding / PEG Placement in Neurodegenerative Disorders

Among the neurodegenerative disorders with progressive dementia, epidemiological data suggest that nutrition can be involved in the aetiopathogenesis of Alzheimer’s disease (AD),

and thus that nutritional intervention can potentially be used in the prevention of the disease. The relative contributions of macro- and micronutrients are not known (24).

The most recent studies suggest benefit of some nutritional compounds to attenuate the neurodegenerative process (e.g. antioxidants and free radical scavengers, omega-3 fatty

acids, Mediterranean diet) (25). This could be of enormous interest in the initial phases of the disease.

On the other hand, deficiencies in zinc and vitamins E and C are associated with cognitive

dysfunction in AD patients, but replacement or supplementation does not lead to improved neurological outcome (26).

Involuntary weight loss is common and may be one of the initial symptoms as AD progresses. The symptoms associated with AD make self-feeding progressively more

difficult; therefore, malnutrition is common. Nutritional status should be assessed at the time of diagnosis, and reassessed during

follow-up. Weight loss or anorexia must alert the caregivers and warrants full nutritional

assessment. We need to plan initial nutritional intervention if weight loss is 5% in 3-6

months. The disturbances in feeding behaviour observed in AD are dominated by anorexia, which

may develop from the onset of the disease. As the disease progresses, feeding behaviour becomes more profoundly disturbed, and can be measured with the Blandford Aversive

Feeding Behaviour Inventory. This scale differentiates four types of disturbances:

- Selective behaviours (refusal to eat a wide range of foods). These disturbances require changes in diet.

- Active behaviours (resistance to feeding, with defensive reflexes)

Factors of the host that affect the defence mechanism to avoid the pass of oropharyngeal secretions and stomach content to the upper airway.

RISK OF BRONCHIAL ASPIRATION & PNEUMONIA

Positioning of patient who

predispose to reflux

Dehydration Level of

conciousness disturbed

Loss of muscle strength

Malnutrition Lower capacity to remove the

aspirated material

Loss of cough reflex

Dysphagia Oropharyngeal colonization

Reduced Control of the tongue

Swallowing capacity delayed or

absent

Immune compromise

?

TO PREVENT BRONCHIAL ASPIRATION

Copyright © by ESPEN LLL Programme 2016

- Oral dyspraxia and attention disorders - Oropharyngeal dysphagia (loss of muscular coordination during mastication and

swallowing, that can lead to repeated problems with swallowing and choking)

In the clinical course of advanced dementia, the prevalence of eating disorders is very high, as is the overall mortality related to eating problems.

Recently, the ESPEN Guidelines on nutrition in dementia have been published (27). In the

guidelines, the different nutritional problems arising from different disease stages are identified (Table 2).

Table 2

Nutritional problems along the stages of dementia

Nutritional problem Stage of dementia

Olfactory and taste dysfunction Preclinical and early stages

Attention deficit Mild to moderate

Executive function deficits (shopping, preparing food) Mild to moderate

Impaired decision-making ability (slowdown in food choice,

reduced intake) Mild to moderate

Dyspraxia (coordination disorders, loss of eating skills) Moderate to severe

Agnosia (Loss of ability to recognize objects or

comprehend the meaning of objects, which means that

food may not be distinguished from non-food and that eating utensils are not recognized as what they are)

Moderate to severe

Behavioural problems Moderate to severe

Oropharyngeal dysphagia Moderate to severe

Refusal to eat Severe

Practical guidelines for the diagnosis and management of weight loss in AD (28)

– A varied and balanced diet and daily physical activity should be recommended for every AD patient to help prevent weight loss.

– Search for reversible medical or socioenvironmental causes of weight loss (including drugs, and especially acetyl cholinesterase inhibitors in the first weeks after the onset

of treatment). – Health-care professionals and family caregivers should follow training centred on the

detection of nutritional risk and on the food/diet of these patients.

– Planning meals (enriched, fractioned, finger foods). – Making the patient drink regularly during the day. Prevention of dehydration is a part

of the management of AD patients. Nourishing drinks are particularly recommended.

Oral supplementation is needed when patients don’t meet their nutritional requirements with an enriched diet. However, patients with advanced AD may be unable or unwilling to

consume an adequate amount of calories by mouth, or may be at risk from dysphagia. In this situation, which can be very distressing for clinicians and families, tube feeding could

be seen as a potential option.

The decision to start tube feeding is one of the most challenging dilemmas. The evidence suggests that it may provide more burden than benefit, and we do not have enough data

available to support tube feeding vs oral feeding in advanced dementia in the prevention of aspiration pneumonia, reduction of the risk of pressure sores, reducing the risk of

infection, improvement in overall function, or in prolonging survival (29).

Copyright © by ESPEN LLL Programme 2016

Moreover, tube feeding represents a considerable use of health care resources. However, we must consider that decisions may and should change with time and clinical situation.

ESPEN guidelines recommend tube feeding for a limited period of time in patients with mild or moderate dementia, to overcome a crisis situation with markedly insufficient oral intake,

if low nutritional intake is predominantly caused by a potentially reversible condition. However, they do not recommend tube feeding once patients have terminal dementia (27).

The use of PEG is also a matter of debate. The indications for placement of PEG tubes should be when they are expected to improve quality of life, reduce episodes of aspiration,

reduce frequency of pressure sores and promote wound healing. However, their benefits have been questioned in patients with advanced dementia. Some studies have shown a

worse prognosis in patients who underwent PEG for advanced dementia, in comparison with other diagnoses that require PEG (30), while other studies have shown no differences

in survival in patients with advanced dementia between those with and without PEG. In the literature there is accordingly some controversy about the placement of PEG tubes in

advanced dementia. Some studies don’t agree that dementia is a negative risk factor for

outcome, but acknowledge poor overall survival (31). Other studies underline the need to design prospective controlled studies (32). Overall, the published data support an

individualized but critical and restrictive approach to PEG feeding in elderly demented patients.

The recommendations should be addressed so as to improve oral intake and avoid aspiration, and to maintain patient comfort and optimise intimate patient care. The concept

of comfort feeding includes careful hand feeding, with an individualized care plan that prioritizes patient comfort, and avoids the misleading dichotomy of care versus no care

(33). The comfort of the patient is of primary importance, even in the setting of weight

loss.

Recently, in the ESPEN Guidelines for Nutrition in Dementia (27), several recommendations have published:

Recommendation Grade of evidence

Strength of

recommendation

We recommend screening every person with dementia for

malnutrition. In case of positive screening, assessment has to follow. In case of positive assessment, adequate

interventions have to follow.

Very Low Strong

We recommend close monitoring and documentation of body

weight in every person with dementia. Very Low Strong

We recommend provision of meals in a pleasant, homelike

atmosphere.

Moderate Strong

We recommend provision of adequate food according to

individual needs with respect to personal preferences.

Very Low Strong

We recommend to encourage adequate food intake and to provide adequate support.

Very Low Strong

We do not recommend the systematic use of appetite

stimulants.

Very Low Strong

We recommend educating caregivers to ensure basic

knowledge on nutritional problems related to dementia and possible strategies to intervene.

Low Strong

We recommend elimination of potential causes of malnutrition as far as possible.

Very Low Strong

Copyright © by ESPEN LLL Programme 2016

We recommend avoiding dietary restrictions. Very Low Strong

We do not recommend the use of omega-3-fatty acid,

vitamin B1, vitamin B6, vitamin B12/folic acid, vitamin E, selenium, copper nor vitamin D supplementation in persons

with dementia for correction of cognitive impairment or

prevention of further cognitive decline.

Different

grades

Strong

We recommend the use of ONS to improve nutritional

status.

High Strong

We do not recommend the use of ONS in persons with dementia to correct cognitive impairment or prevent further

cognitive decline.

Moderate Strong

We do not recommend the systematic use of special medical

foods for persons with dementia to correct cognitive impairment or prevent further cognitive decline.

Low Strong

We do not recommend any other nutritional product for persons with dementia to correct cognitive impairment or

prevent further cognitive decline.

Very Low Strong

We recommend that each decision for or against artificial nutrition and hydration for patients with dementia is made

on an individual basis with respect to general prognosis and

patients' preferences.

Very Low Strong

We suggest tube feeding for a limited period of time in

patients with mild or moderate dementia, to overcome a crisis situation with markedly insufficient oral intake, if low

nutritional intake is predominantly caused by a potentially reversible condition.

Very Low Weak

We recommend against the initiation of tube feeding in

patients with severe dementia.

High Strong

We suggest parenteral nutrition as an alternative if there

is an indication for artificial nutrition, as described in

recommendation 16, but tube feeding is contraindicated or

not tolerated.

Very Low Weak

We suggest parenteral fluids for a limited period of time

in periods of insufficient fluid intake to overcome a crisis situation.

Very Low Weak

We recommend against the use of artificial nutrition (enteral nutrition, parenteral nutrition and parenteral fluids)

in the terminal phase of life.

Very Low Strong

5. Ethical Considerations around Tube Feeding in Patients with

Neurodegenerative Disorders with Advanced Dementia

Consent obtained from the patient, if he or she is competent, is necessary before commencing tube feeding. But if the patient is not competent, the consent must be

obtained from the individual with legal power of attorney. In some jurisdictions this will be

the next of kin, but it may also be the attending physician. In all circumstances when the patient is not competent, the physician has to act in the patient’s best interest, and the

effects on quality of life and functional status may be the most relevant outcomes to be considered (34).

Copyright © by ESPEN LLL Programme 2016

Even if no advanced directive is available, we need to give priority to the previously expressed wishes of the patient, and be guided by what it is believed the patient would

choose if they were competent, always following the patient’s best interest. If there is a disagreement, we can consider approaching a Clinical Ethical Committee for

advice. The establishment of such a committee in a growing number of healthcare institutions will facilitate this process.

Legally and ethically, Specialized Nutritional Support (SNS) should be considered a medical

therapy. For this reason, adult patients or their legally authorized surrogates have the right to accept or to refuse SNS. The benefits and burdens of SNS should be considered before

offering this therapy, and institutions should develop clear policies regarding withdrawal from or withholding SNS.

Recently, the ESPEN Guidelines on ethical aspects on artificial nutrition and hydration (35) has reached strong consensus on the following recommendations:

The will of the adult patient who is capable of providing consent and making judgments

has to be respected in any case.

The patient or his or her authorized representative should be informed about the

nature, significance and scope of the measure, including potential complications and

risks.

In the case that a patient is unable to give consent and make judgments, the

representative (authorized according to different rules depending on the country’s law

and practice) takes the decision. The representative has to implement the presumed

will of the patient. If the representatives' decision is delayed, the physician should start

artificial nutrition according to evidence based medical indications.

Quality of life must always be taken into account in any type of medical treatment

including artificial nutrition.

6. Summary Chronic neurological patients are a heterogeneous group including those in the chronic

phase of an acute injury (acute stroke, brain trauma, and spinal cord injury) and also patients with chronic neurodegenerative diseases, some of them with a progressive and

fatal course. Malnutrition is a risk factor for increased morbidity and mortality, and nutritional support plays an important role in the management of all of these diseases.

Dysphagia is the major concern that should be identified and treated as early as possible. If dysphagia is severe or cannot be managed with dietary counselling or modified

consistency food, enteral tube feeding should be offered to the patient.

In neurodegenerative disorders such as multiple sclerosis or amyotrophic lateral sclerosis, the most important concerns will be the management of dysphagia and the decision to

start enteral nutrition via a PEG. Amyotrophic lateral sclerosis patients generate the most frequent dilemmas concerning timing/efficacy and security of PEG placement.

In neurodegenerative disorders with advanced dementia, there are no data available to support tube feeding versus oral feeding. Patients with advanced dementia are not usually

considered appropriate candidates for artificial feeding via PEG tubes. Legally and ethically, specialized nutritional support should be considered a medical

therapy, and advanced directives can help us to resolve conflicts over treatments.

Copyright © by ESPEN LLL Programme 2016

7. References 1. Cook AM, Hatton J. Neurological impairment. In: ASPEN Nutrition Support Core

Curriculum: A case-based approach- The Adult Patient. Gottschlich MM, ASPEN editions 2007: 424-439.

2. Scheard JM. Malnutrition and neurodegenerative diseases. Curr Opin Nutr Rep 2014;

3: 102-9. 3. Pasquinelli S, Solaro C. Nutritional assessment and malnutrition in multiple sclerosis.

Neurol Sci 2008;29 Suppl 4:S367-9. 4. Payne A. Nutrition and diet in the clinical management of multiple sclerosis. J Hum

Nutr Dietet 2001; 14: 349-357. 5. James E, Dobson R, Kuhle J, Baker D, Giovannoni G, Ramagopalan SV. The effect of

vitamin D-related interventions on multiple sclerosis relapses: a meta-analysis. Mult Scler 2013. 19(12):1571-9.

6. Schwarz S, Leweling H. Multiple sclerosis and nutrition. Multiple Sclerosis 2005; 11:

24-32. 7. Solaro C, Rezzani C, Trabucco E, Amato MP, Zipoli V, Portaccio E et al. Prevalence of

patient-reported dysphagia in multiple sclerosis patients: an Italian multicenter study (using the DYMUS questionnaire). J Neurol Sci. 2013;331(1-2):94-7.

8. Giusti A1, Giambuzzi M. Management of dysphagia in patients affected by multiple sclerosis: state of the art. Neurol Sci. 2008 Dec;29 Suppl 4:S364-6.

9. Poorjavad M1, Derakhshandeh F, Etemadifar M, Soleymani B, Minagar A, Maghzi AH. Oropharyngeal dysphagia in multiple sclerosis. Mult Scler. 2010 Mar;16(3):362-5.

10. Thomas FJ, Wiles CM. Dysphagia and nutritional status in multiple sclerosis. J Neurol

1999; 246: 677-682. 11. Cushing ML, Traviss KA, Calne SM. Parkinson’s disease: Implications for Nutritional

Care. Can J Diet Prac Res 2002; 63: 81-87. 12. Barichella M1, Cereda E, Pezzoli G. Major nutritional issues in the management of

Parkinson's disease. Mov Disord. 2009 Oct 15;24(13):1881-92. 13. Sheard JM, Ash S, Mellick GD, Silburn PA, Kerr GK. Markers of disease severity are

associated with malnutrition in Parkinson's disease. PLoS One. 2013;8(3):e57986. 14. Antonini A, Ursino G, Calandella D, Bernardil L, Plabani M. Continous dopaminergic

delivery in Parkinson’s disease. J Neurol 2010: 257 (Suppl 2): S305-8.

15. Cereda E. Barichella M, Pedrolli C, Pezzoli G. Low protein and protein redistribution diets for Parkinson’s Disease patients with motor fluctuations: A Systematic Review.

Mov Dis 2010. 16. Chiò A, Logroscino G, Hardiman O, Swingler R, Mitchell D, Beghi E, et al. Prognostic

factors in ALS: A critical review. Amyotroph Lateral Scler. 2009 Oct-Dec;10(5-6):310-23.

17. Muscaritoli M, Kushta I, Molfino A, Inghilleri M, Sabatelli M, Rossi Fanelli F. Nutritional and metabolic support in patients with amyotrophic lateral sclerosis. Nutrition

2012;28(10):959-66.

18. Genton L1, Viatte V, Janssens JP, Héritier AC, Pichard C. Nutritional state, energy intakes and energy expenditure of amyotrophic lateral sclerosis (ALS) patients. Clin

Nutr 2011;30(5):553-9. 19. Cameron A, Rosenfeld J. Nutritional issues and supplements in amyotrophic lateral

sclerosis and other neurodegenerative disorders. Curr Opin Clin Nutr Metab Care 2002; 5: 631-643.

20. Katzberg HD, Benatar M.Enteral tube feeding for amyotrophic lateral sclerosis/motor

neuron disease. Cochrane Database of Systematic Reviews 2011, Issue 1. Art. No.:

CD004030. DOI: 10.1002/14651858.CD004030.pub3. 21. National Clinical Guideline Centre. NICE guideline NG42: Motor neurone disease:

assessment and management. Methods, evidence and recommendations. February 2016.

22. Miller RG, Jackson CE, Kasarkis EJ, England JD, Forsheno D, Johnston W et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug,

Copyright © by ESPEN LLL Programme 2016

nutritional and respiratory therapies (an evidence-based review) Neurology 2009; 73: 1218-26.

23. DeLegge MH. Enteral nutrition and the neurologic diseases. In: Rolandelly RH. Enteral and Tube Feeding, 4th edition. Elsevier Ed, 2005: 406-413.

24. Steele M, Stuchbury G, Münch G. The molecular basis of the prevention of Alzheimer’s disease through healthy nutrition. Experimental Gerontol 2007; 42: 28-36.

25. Staehelin H. Micronutrients and Alzheimer’s disease. Proc Nutr Soc 2005; 64: 565-70.

26. Planas M, Conde M, Audivert S, Pérez-Portabella C, Burgos R, Chacón P et al. Micronutrient supplementation in mild Alzheimer disease patients. Clin Nutr 2004; 23:

265-72. 27. Volkert D, Chourdakis M , Faxen-Irving G , Fruhwald T, Landi F, Suominen MH et al.

ESPEN guidelines on nutrition in dementia, Clinical Nutrition (2015), http://dx.doi.org/ 10.1016/j.clnu.2015.09.004.

28. Belmin J. Expert Panel and Organization Committee. Practical guidelines for the diagnosis and management of weight loss in Alzeimer’s disease: a consensus from

appropriateness ratings of a large expert panel. J Nutr Health Aging 2007; 11 (1): 33-7.

29. Sampson EL, Candy B, Jones L. Enteral tube feeding for older people with advanced dementia. Cochrane Database of Systematic reviews 2009; Issue 2: CD007209.

30. Sander DS, Carter MJ, James G, Bottom RP, Bardhan KD. Survival analysis

inpercutaneous endoscopic gastrostomy feeding: a worse outcome in patients with dementia. Am J Gastroenterol 2000; 95 (6): 1472-5.

31. Gaines DI, Dukalski V, Patel A, DeLegge MH. Dementia and cognitive impairment are not associated with earlier mortality after PEG. JPEN 2009; 33 (1): 62-6.

32. Freeman C, Ricevuto A, DeLegge MH. Enteral nutrition in patients with dementia and stroke. Curr Opin Gastroenterol 2010; 26 (2): 156-9.

33. Palecek EJ, Teno JM, Casarett DJ, Hanson LC, Rhodes RL, Mitchell SL. Comfort feeding only: a proposal to bring clarity to decision-making regarding difficulty with eating for

persons with advanced dementia. J Am Geriatr Soc 2010; 58 (3): 580-4.

34. Körner U, Bondolfi A, Bühler E, MacFie J, Meguid MM, Messing B et al. Ethical and legal aspects of enteral nutrition. Clin Nutr 2006; 25: 196-202.

35. Druml C, ESPEN guideline on ethical aspects of artificial nutrition and hydration. Clin Nutr 2016, http://dx.doi.org/10.1016/j.clnu.2016.02.006